Section I Tetralogy of Fallot with Pulmonary Stenosis Definition Tetralogy of Fallot (TF) is a congenital cardiac malformation characterized by underdevelopment of the right ventricular (RV) infundibulum, with anterior and leftward displacement of the infundibular (conal, outlet) septum and its…
Definition Patent ductus arteriosus (PDA) is abnormal persistence of a patent lumen in the fetal ductus arteriosus, which usually connects the upper descending thoracic aorta with the proximal portion of the left pulmonary artery (LPA). When the aortic arch is…
Section I Unruptured and Ruptured Sinus of Valsalva Aneurysms Definition Congenital sinus of Valsalva aneurysms are thin-walled saccular or tubular outpouchings usually located in the right sinus or adjacent half of the noncoronary sinus. They generally have an intracardiac course…
Section I Primary Ventricular Septal Defect Definition Ventricular septal defect (VSD) is a hole or multiple holes in the interventricular septum. This chapter discusses VSDs that occur as the primary lesion, recognizing that hearts with primary VSDs may have minor…
Definition Atrioventricular (AV) septal defects are characterized by a deficiency or absence of septal tissue immediately above and below the normal level of the AV valves, including the region normally occupied by the AV septum, in hearts with two ventricles.…
Definition Unroofed coronary sinus syndrome is a spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and left atrium is absent. Hearts with atrial isomerism and a left-sided superior vena cava (LSVC)…
Definition Classic (or typical) cor triatriatum, or cor triatriatum sinister, is a rare congenital cardiac anomaly in which the pulmonary veins typically enter a “proximal” left atrial chamber separated from the “distal” left atrial chamber by a partition in which…
Definition Total (totally) anomalous pulmonary venous connection (TAPVC) is a cardiac malformation in which there is no direct connection between any pulmonary vein and the left atrium; rather, all the pulmonary veins connect to the right atrium or one of…
Definition An atrial septal defect (ASD) is a hole of variable size in the atrial septum. A patent foramen ovale that is functionally closed by overlapping of limbic tissue superiorly and the valve of the fossa ovalis inferiorly (in response…
Definition Congenital heart disease in the adult is presence of unrepaired or repaired congenital heart disease in patients aged 21 years or older. A more practical working definition is not straightforward. Because physical and emotional maturity is variable, the distinction…